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Acute and Chronic Liver Disease B - Coggle Diagram
Acute and Chronic Liver Disease B
Primary biliary (cholangitis) cirrhosis
Characteristics
Autoimmune disease of middle-aged women, associated with other autoimmune disorders
Risk Factors + Pathogenesis
Genetic Predisposition + Environmental Trigger
Dysregulated innate and adaptive immune against mitochondrian antigens within cholangiocytes
Symtpoms
Fatigue, Pruritus, Many asymptomatic, dry eyes, dry mouth, xanthoma, xanthelasma, Low bone mass
Investigations
Increase in ALP, AST/ALT/Bilirubin Normal
+AMA
Increased IgM
Diagnosis
2/3 Of criteria: Elevated ALP, AMA positive, Histology with PBC
Treatment
UDCA (secondary bile acid), Obethicolic Acid, Off-label second line therapy, therapies to control symptoms, transplantation for liver failure
Primary sclerosing cholangitis
Characteristics
70% men with average age of 40, 70% of PSC patients have ulcerative colitis
Pathogenesis
Autoimmune damage of medium size intra- and extra- hepatic bile ducts
Inflammation -> Progressive fibrosis -> Stenosis and dilatation of biliary tree
Clinical Symtpoms
Fatigue, Pruritus
Cholangitis, Steatorrhea lack of vitamins A, D, E, K
Lab Tests
Increase in ALP, mild increase in AST/ALT
Bilirubin may be elevated
Increased IgM
Imaging/Investigations
Abdo US
Biliary Tree thickening/Stricture
CT Scan
Intrahepatic Bile duct Thickening
Cholangiopancreatography
ERCP, MRCP, "Beaded Appearance"
Liver Biopsy
Onion Skin Fibrosis
Treatment
Liver Transplant, no effective treatment, ERCP dilatation of tight strictures,
Wilson's Disease
Pathophysiology
Abnormality in handling of copper - Cannot excrete copper out of liver
Copper deposits in liver and basal ganglia
Presentation
Must be ruled out in all young patients
ABN LFTs, Fulminant Hepatic Failure, Chronic Active hepatitis, Cirrhosis
CNS Symtoms: Ataxia, dystonia, tremor, dysarthria, parkinsonian syndrome, depression
Investigations
Decreased ceruloplasmin (copper binding agent) in blood, Increased urinary copper, Kayser Fleisher rings
Treatment
D-Penicillamine - Copper chelator, Trientene (copper chelator), Oral zinc interferes with GI copper absorption, Liver Transplant
Hemochromatosis
Characteristics
Common Inherited Disorders
Excessive iron absorption in gut, deposition in liver, heart, joints, pancreas, and other endocrine organs
Clinical Presentation
Raised Ferritin, ALT, high Iron saturation
Other Organ Damage: Arthritis, Cardiomyopathy, skin pigmentation, diabetes, hypothyroidism, testicular atrophy
Treatment
Gene Testing, Phlebotomy, family screening, Hepatoma
Budd-Chiari
Characteristics
Blockage of Hepatic Vein
Usually secondary to hypercoagulable state
Presentation
Ascites/Hepatomegaly
Ps: Pills, pregnancy, polycythemia
Diagnsosi
US/Doppler of hepatic veins
Treatment
Anticoagulant +/- TIPS +/- Liver Transplant
Autoimmune hepatitis
Characteristics
Disease of young women and menopause women
Pathogenesis
Precipitating factors: virus or medications
Loss of immune tolerance to hepatocytes leading to destruction of hepatic cells leads to hepatocellular damage and progressive fibrosis
Clinical features
Asymptomatic, fatigue, Fluctuating ALT/AST elevation
Liver failure/cirrhosis
Diagnosis
High AST, ALT may be elevated, IgG is elevated, ANA +ve, anti-smooth muscle antibody, Liver biopsy showing chronic hepatitis
Treatment
Corticosteroids +/- Imuran
Alpha-1-antitrypsin deficiency
Characteristics
Deficiency in alpha-1 antitrypsin
Inhibits proteases, Made in liver to act on lung
Causes apoptosis, liver cirrhosis
In lung - No elastase and leads to emphysema
Autosomal Recessive
Liver disease is associated with PiZZ phenotype
Mutant Z allele encodes structural change in glycoprotein
Cholestasis
Etiology
Formation or excretion of bile is impaired
Symptoms
asymptomatic, fatigue, pruritus, abdominal pain, scleral icterus
Investigations
Increase in ALP, Increase GGT, Increase billirubin
Reviews meds, non prescription, herbals
Abdo U/S
MRCP, ERCP
Serology: AMA, ANA< SMA, Ig, IgG4, ACE levels
Acute vs Chronic
Intra vs Extrahepatic vs mixed
Intrahepatic causes
Sepsis, viral hepatitis, alcoholic hepatitis, non-alcoholic hepatitis, cirrhosis
Infiltrative: Amyloidosis, sarcoidosis, lymphoma
Vascular: Budd Chiari Syndrome, Congestive hepatopathy (Right sided heart failure)
IgG4 disease
Extrahepatic causes
Benign
Choledocholithiasis, Pancreatitis, HIV Cholangiopathy
Malignant
Cholangiocarcinoma, Pancreatic cancer (head), Ampullary cancer
